AB0674 PRIMARY SJOGREN SYNDROME WITH ANTICENTROMERE ANTIBODIES - A CLINICALLY DISTINCT PHENOTYPE OR AN OVERLAP SYNDROME?
Authors: N. Cristina, D. Nicoara, L. Groseanu, D. Opris Belinski, V. Bojinca, D. Predeteanu, F. Berghea, V. Vlad, I. Saulescu, S. Daia-Iliescu, D. Mazilu, A. Borangiu, C. Cobilinschi, M. M. Negru, C. L. Constantinescu, D. Mardale, M. Duna, M. Abobului, R. Ionescu, A. Balanescu
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Anticentromere antibodies (ACA) have been reported in primary Sjogren syndrome (pSS) at lower frequencies but have recently been suggested as another clinically important marker in this disease<sup>1</sup>. Although several papers have been published on ACA-positive pSS, the results have been conflicting<sup>2,3</sup>. <h3>Objectives</h3> To evaluate the correlation between presence of ACA and associated symptomatology, especially related to microvascular dysfunction in a Romanian cohort of patients with ACA-positive pSS. <h3>Methods</h3> We assessed 132 patients who met the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for pSS. For all ACA+ patients, we collected data on the presence of sclerodactyly, puffy hands, Raynaud's phenomenon, calcinosis, telangiectasia, esophageal involvement, interstitial lung disease, digital ulcerations/pitting scars, pulmonary hypertension and nailfold capillaroscopy findings. <h3>Results</h3> The prevalence of ACA in our pSS cohort was 13%. ACA - positive pSS patients were older than the ACA-negative patients (median, 59 years [IQR, 48 to 62] vs median, 50 years [IQR, 45 to 60]; p = 0.042), with a shorter disease duration (53 months vs 260 months, p=0.073). Most patients were female (98.7%) and had ocular and oral symptoms of dryness (98.1% and 97.8%, respectively). Among the patients with pSS, 5 patients had ACA and limited cutaneous systemic sclerosis (lcSSc). Although there was no statistical differences between the two groups, ACA-positive patients were characterized by higher prevalence of associated symptomatology related to microvascular dysfunction: RP (Raynaud's phenomenon) and telangiectasias were present in 50% of the patients (5/10), sclerodactyly and digital ulcerations (DUs) in 33% (3/10); digital gangrene was not found. 60% (6/10) of ACA+ pSS patients had abnormal findings on nailfold capillaroscopy, which were nonspecific in 2 patients (crossed capillaries), while in the remaining 1 patient had an early-pattern, 1 had an active-pattern and 2 patients had late scleroderma-pattern. The presence of ACA was positively correlated with the presence of RP (p = 0.024), DUs (p = 0.034). In our study, patients showed similar severities in extraglandular manifestations compared to the ACA-negative group. Nonetheless, we noticed that even if not statistically significant, the use of vasodilators was more common in the ACA - positive pSS patients (66.7% vs 12.2%, p = 0.911) thus supporting the hypothesis of a symptomatic microvascular dysfunction. A higher proportion of ACA+ pSS (64% vs 42%) was treated with immunosuppressive agents. No differences in mortality were found between the groups. <h3>Conclusion</h3> Patients in the ACA-positive group were older, had more frequent RP and digital ulcers. In our cohort, ACA - positivity had little impact on the severity of extraglandular manifestations in pSS patients. Although there were no significant differences, use of vasodilators and immunosuppressants agents was more common. This study is the first to investigate the impact of ACA positivity on the vascular manifestations of Romanian pSS patients. <h3>References</h3> [1]Youngjae Park Clinical influences of anticentromere antibody on primary Sjögren's syndrome in a prospective Korean cohort Korean J Intern Med 2021;36(6):1492-1503. [2]Nakamura H, Kawakami A, Hayashi T, et al. Anti-centromere antibody-seropositive Sjogren's syndrome differs from conventional subgroup in clinical and pathological study. BMC Musculoskelet Disord 2010;11:140. [3]Baer AN, Medrano L, McAdams-DeMarco M, Gniadek TJ. Association of anticentromere antibodies with more severe exocrine glandular dysfunction in Sjogren's syndrome: analysis of the Sjogren's International Collaborative Clinical Alliance Cohort. Arthritis Care Res (Hoboken) 2016;68:1554-1559. <h3>Disclosure of Interests</h3> None declared